目的:报告1例伴有HIP1/PDGFRB融合基因的不典型慢性增殖性髓系肿瘤,并就第4版WHO造血及淋巴组织肿瘤提出的新的一类伴PDGFRB基因异常髓系肿瘤进行文献综述。方法:患者脾大、乏力15年,病程中先后出现红细胞和血小板增多、巨脾、贫血、白细胞增多、单核细胞及嗜酸粒细胞增多。结果:骨髓细胞HIP1/PDGFRB融合基因检查为阳性,酪氨酸激酶抑制剂伊马替尼治疗有效。结论:酪氨酸激酶抑制剂伊马替尼治疗伴有HIP1/PDGFRB融合基因的髓系肿瘤临床效果满意。 OBJECTIVE: To report a case of atypical chronic myeloid neoplasm associated with HIP1 / PDGFRB fusion gene and review the literature review of a new class of PDGFRB gene myeloid neoplasms proposed by the 4th edition of WHO hematopoietic and lymphoid tumors. Methods: Patients with splenomegaly, fatigue 15 years, the course of the disease has appeared red blood cells and thrombocytosis, spleen, anemia, leukocytosis, mononuclear cells and eosinophilia. Results: HIP1 / PDGFRB fusion gene was positive in bone marrow cells and imatinib tyrosine kinase inhibitor was effective. Conclusions: The efficacy of tyrosine kinase inhibitor imatinib in treatment of myeloid neoplasms associated with HIP1 / PDGFRB fusion gene is satisfactory.